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Autophagy induction reduces mutant ataxin-3 levels and toxicity in a mouse model of spinocerebellar ataxia type 3

Spinocerebellar ataxia type 3 is a neurodegenerative disorder caused by the expansion of the polyglutamine repeat region within the ataxin-3 protein. The mutant protein forms intracellular aggregates in the brain. However, the cellular mechanisms causing toxicity are still poorly understood and ther...

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Detalles Bibliográficos
Autores principales: Menzies, Fiona M., Huebener, Jeannette, Renna, Maurizio, Bonin, Michael, Riess, Olaf, Rubinsztein, David C.
Formato: Texto
Lenguaje:English
Publicado: Oxford University Press 2010
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2801325/
https://www.ncbi.nlm.nih.gov/pubmed/20007218
http://dx.doi.org/10.1093/brain/awp292